Literature DB >> 25465280

Treatment responsive GABA(B)-receptor limbic encephalitis presenting as new-onset super-refractory status epilepticus (NORSE) in a deployed U.S. soldier.

Jeffrey Brian Hainsworth1, Akira Shishido2, Brett James Theeler3, Craig Grason Carroll1, Rebecca Ellen Fasano1.   

Abstract

A 23-year-old, previously healthy, deployed U.S. soldier presented with bilateral temporal lobe seizures recalcitrant to multiple antiepileptic drugs and anti-seizure anaesthetic agents. He received methylprednisolone, intravenous immunoglobulins, plasma exchange, and rituximab for presumed autoimmune encephalitis before achieving seizure freedom. Six weeks after presentation, the aetiology of his refractory seizures was found to be due to autoantibodies targeting the anti-GABA(B)-receptor. This case is noteworthy for being the first reported case of anti-GABA(B)-receptor limbic encephalitis presenting with new-onset refractory status epilepticus (NORSE), a clinical syndrome that often carries a grave prognosis and in which a treatable aetiology is often never discovered. Our case also supports testing for GABA-receptor autoantibodies and the upfront use of multi-modal immunotherapy in patients presenting with limbic encephalitis and new refractory seizures.

Entities:  

Keywords:  GABA(B)-receptor; all epilepsy/seizures; encephalitis; new-onset refractory status epilepticus (NORSE); status epilepticus

Mesh:

Substances:

Year:  2014        PMID: 25465280     DOI: 10.1684/epd.2014.0702

Source DB:  PubMed          Journal:  Epileptic Disord        ISSN: 1294-9361            Impact factor:   1.819


  4 in total

1.  New-onset refractory status epilepticus: Etiology, clinical features, and outcome.

Authors:  Nicolas Gaspard; Brandon P Foreman; Vincent Alvarez; Christian Cabrera Kang; John C Probasco; Amy C Jongeling; Emma Meyers; Alyssa Espinera; Kevin F Haas; Sarah E Schmitt; Elizabeth E Gerard; Teneille Gofton; Peter W Kaplan; Jong W Lee; Benjamin Legros; Jerzy P Szaflarski; Brandon M Westover; Suzette M LaRoche; Lawrence J Hirsch
Journal:  Neurology       Date:  2015-08-21       Impact factor: 9.910

2.  Cryptogenic NORSE: Its distinctive clinical features and response to immunotherapy.

Authors:  Takahiro Iizuka; Naomi Kanazawa; Juntaro Kaneko; Naomi Tominaga; Yutaka Nonoda; Atsuko Hara; Yuya Onozawa; Hiroki Asari; Takashi Hata; Junya Kaneko; Kenji Yoshida; Yoshihiro Sugiura; Yoshikazu Ugawa; Masashi Watanabe; Hitomi Tomita; Arifumi Kosakai; Atsushi Kaneko; Daisuke Ishima; Eiji Kitamura; Kazutoshi Nishiyama
Journal:  Neurol Neuroimmunol Neuroinflamm       Date:  2017-09-25

3.  Limbic Encephalitis Associated with Anti-γ-aminobutyric Acid B Receptor Antibodies: A Case Series from China.

Authors:  Hong-Zhi Guan; Hai-Tao Ren; Xun-Zhe Yang; Qiang Lu; Bin Peng; Yi-Cheng Zhu; Xiao-Qiu Shao; Yong-Qiang Hu; Dong Zhou; Li-Ying Cui
Journal:  Chin Med J (Engl)       Date:  2015-11-20       Impact factor: 2.628

Review 4.  [Pharmacotherapy and intensive care aspects of status epilepticus: update 2020/2021].

Authors:  Gabrielė Saitov; Annekatrin Müller; Börge Bastian; Dominik Michalski
Journal:  Anaesthesist       Date:  2021-07-01       Impact factor: 1.041

  4 in total

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